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第 274 届 ENMC 国际研讨会:优化神经肌肉疾病中骨骼强度的建议。荷兰霍夫多普,2024 年 1 月 19-21 日。

274th ENMC international workshop: recommendations for optimizing bone strength in neuromuscular disorders. Hoofddorp, The Netherlands, 19-21 January 2024.

机构信息

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.

Department of Pediatrics, Radboud University Medical Center, Radboudumc Amalia Children's Hospital, Nijmegen, the Netherlands.

出版信息

Neuromuscul Disord. 2024 Oct;43:1-13. doi: 10.1016/j.nmd.2024.07.009. Epub 2024 Aug 21.

Abstract

The 274th ENMC workshop for optimizing bone strength in neuromuscular disorders (NMDs) was held on January 19-21, 2024. The group of participants included experts in the fields of bone health and neuromuscular medicine along with the patient voice. Bone strength represents a crucial aspect of the management of pediatric and adult patients with NMDs. Bone strength may be compromised due to different pathophysiologic mechanisms, including disrupted bone-muscle "cross-talk", loss of biomechanical loading, nutritional insufficiency, inadequate weight-bearing physical activity, muscle weakness and/or immobility, and drug treatment. While for Duchenne muscular dystrophy recommendations for evaluation and treatment of bone strength have been published, evidence on bone strength in other hereditary and acquired NMDs is scarce. Enhanced knowledge is needed to understand the development and maintenance of bone strength in patients with NMDs. This workshop aimed to develop a strategy to improve bone strength and thus prevent fractures in patients with NMDs.

摘要

第 274 届 ENMC 优化神经肌肉疾病(NMDs)患者骨强度研讨会于 2024 年 1 月 19 日至 21 日举行。与会者包括骨骼健康和神经肌肉医学领域的专家以及患者代表。骨强度是管理儿科和成年 NMD 患者的关键方面。由于不同的病理生理机制,包括骨骼-肌肉“对话”中断、生物力学负荷丧失、营养不足、缺乏负重体力活动、肌肉无力和/或活动受限以及药物治疗等,骨强度可能受损。虽然已经发表了针对杜氏肌营养不良症患者骨强度评估和治疗的建议,但其他遗传性和获得性 NMD 患者的骨强度证据很少。需要增强知识以了解 NMD 患者骨强度的发展和维持。本次研讨会旨在制定一项改善骨强度的策略,从而预防 NMD 患者骨折。

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